{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T10:27:47Z","timestamp":1761560867924},"reference-count":16,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,2,18]],"date-time":"2004-02-18T00:00:00Z","timestamp":1077062400000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2004,2]]},"abstract":"<jats:title>Abstract:<\/jats:title><jats:p>Initial diagnosis of cavernous transformation of the portal vein (portal cavernoma) is rarely made in adults. Its main clinical manifestation is upper gastrointestinal hemorrhage due to variceal bleeding. More rarely, diagnosis is made from obstructive jaundice. In children, this condition is frequently associated to prehepatic portal hypertension and congenital anomalies, the most frequent of which are atrial septal defects or malformations of the biliary tract or of the inferior vena cava.<\/jats:p><jats:p>We describe here a case of a 23\u2010year\u2010old female presenting with massive hematemesis due to the presence of esophageal and small intestinal varices. She had a cavernous transformation of the portal vein with prehepatic portal hypertension associated with heretofore unreported malformations such as right pulmonary hypoplasia, cardiac dextroposition, and right renal ectopia. A unifying hypothesis (e.g. an intrauterine vascular insult) to explain the pathogenesis of these defects seems unlikely. Appropriate tests failed to identify specific functional abnormalities in these organs. Although she bled more than once, the combination of sclerotherapy and \u03b2\u2010blockers has been, thus far, able to control the major clinical consequences of this disease.<\/jats:p>","DOI":"10.1111\/j.1478-3231.2004.00890.x","type":"journal-article","created":{"date-parts":[[2004,2,18]],"date-time":"2004-02-18T16:37:32Z","timestamp":1077122252000},"page":"80-83","source":"Crossref","is-referenced-by-count":11,"title":["Cavernous transformation of the portal vein associated to multiorgan developmental abnormalities"],"prefix":"10.1111","volume":"24","author":[{"given":"D.","family":"Sorrentino","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Labombarda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"Debiase","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Trevisi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Giagu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,2,18]]},"reference":[{"key":"e_1_2_4_2_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2002-23206"},{"key":"e_1_2_4_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/bjs.1800560105"},{"key":"e_1_2_4_4_2","doi-asserted-by":"publisher","DOI":"10.1259\/0007-1285-51-612-953"},{"key":"e_1_2_4_5_2","doi-asserted-by":"publisher","DOI":"10.1259\/0007-1285-51-612-1011"},{"key":"e_1_2_4_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9610(05)80881-5"},{"key":"e_1_2_4_7_2","first-page":"627","article-title":"The aetiology, presentation and natural history of extrahepatic portal venous obstruction","volume":"192","author":"Webb L J","year":"1979","journal-title":"Q J Med"},{"key":"e_1_2_4_8_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.52.5.383"},{"key":"e_1_2_4_9_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-1009120"},{"issue":"9","key":"e_1_2_4_10_2","first-page":"1476","article-title":"Cavernous transformation of the portal vein","volume":"90","author":"Bayraktar Y","year":"1995","journal-title":"a common manifestation of Beh\u00e7et's disease"},{"key":"e_1_2_4_11_2","first-page":"1088","volume-title":"Textbook of Surgery: The Biological Basis of Modern Surgical Practice","author":"Rikkers L F.","year":"1997"},{"key":"e_1_2_4_12_2","first-page":"3259","article-title":"Diseases of the hepatic blood vessels","author":"Bolt R J.","year":"1985","journal-title":"Bockus Gastroenterol"},{"key":"e_1_2_4_13_2","first-page":"439","article-title":"Extrahepatic portal obstruction. Clinical experiences and surgical treatment in 105 patients","volume":"21","author":"Belli L","year":"1980","journal-title":"J Cardiovasc Surg"},{"key":"e_1_2_4_14_2","first-page":"777","article-title":"Treatment of extrahepatic portal hypertension in children by mesenteric\u2010to\u2010left portal vein bypass","volume":"165","author":"De Ville de Goyet J","year":"1999","journal-title":"a new physiological procedure"},{"issue":"1","key":"e_1_2_4_15_2","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/S0022-3468(00)80005-6","article-title":"Experience with the Rex shunt (mesenterico\u2010left portal bypass) in children with extrahepatic portal hypertension","volume":"35","author":"Bambini D A","year":"2000","journal-title":"J Pediatr Surg"},{"issue":"3","key":"e_1_2_4_16_2","first-page":"137","article-title":"Endoscopic sclerotherapy in children with extrahepatic portal venous obstruction","volume":"22","author":"Patraval V","year":"2001","journal-title":"Trop Gastroenterol"},{"issue":"2","key":"e_1_2_4_17_2","first-page":"101","article-title":"Portal cavernoma associated with agenesis of the left kidney","volume":"7","author":"Esposito G.","year":"1966","journal-title":"Ann Chir Infant"}],"container-title":["Liver International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1478-3231.2004.00890.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1478-3231.2004.00890.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,14]],"date-time":"2023-10-14T15:23:49Z","timestamp":1697297029000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1478-3231.2004.00890.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,2]]},"references-count":16,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2004,2]]}},"alternative-id":["10.1111\/j.1478-3231.2004.00890.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1478-3231.2004.00890.x","archive":["Portico"],"relation":{},"ISSN":["1478-3223","1478-3231"],"issn-type":[{"value":"1478-3223","type":"print"},{"value":"1478-3231","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,2]]}}}